[Diagnosis and treatment of orbit solitary fibrous tumor: a report of 4 cases].

OBJECTIVE: To investigate the diagnosis, treatment and prognosis of orbital solitary fibrous tumor. METHODS: Clinical data of 4 cases with orbital solitary fibrous tumor from January 2001 to June 2014 in the Second Affiliated Hospital of Xi'an Jiaotong University was retrospectively analyzed and the image, pathologic and immunohistochemical findings were reviewed. RESULTS: In the 4 cases, 3 were males and 1 was female, aged from 48 to 67 years. The main symptoms were unilateral progressive proptosis, orbital tumor and decreased vision. Two cases involved the left orbit and 2 in right. The locations of the tumor were in the lateral (2 cases) or inferior orbit (2 cases). Encapsulated smooth round shadow was shown in imaging examination and a homogeneous enhancement strengthening was seen by CT scanning. All cases underwent surgical resection and the removed tumors, appeared as round or irregular oval with fibrous capsule, were 1.5-5.0 cm in size. Three cases were pathological benign and 1 was malignant. Microscopically, the tumors were composed of a large number of spindle tumor cells and varying amounts of interstitial collagen deposition and angiogenesis. There was no atypia in benign tumor, while there was atypia in malignant tumor. Moreover, the tumor was invasive, capsuleless and shown hyperplasia area by light microscope in the case with malignant disease. CD34 and Vimentin were positive in 4 cases and Bcl-2 positive in 2 cases by immunohistochemistry staining. One patient with malignant pathology showed strong positive staining of Ki-67 (>70%) and died of tumor recurrence 10 months after he received the second operation. No metastasis or recurrence occurred by a follow-up of 2 months to 5 years in other 3 cases. CONCLUSIONS: Orbital SFT is characteristic of unilateral progressive proptosis in symptom. Imaging examination is an important diagnostic tool and a complete surgical resection of the tumor is the primary treatment method. Diagnosis depends on pathological and immunohistochemical staining. Malignant transformation may be occurred from benign lesion, hence postoperative follow-up is essential for confirmed patients.(Chin J Ophthalmol, 2016, 52: 268-272).

Copper transportion of WD protein in hepatocytes from Wilson disease patients in vitro.

AIM: To study the effect of copper transporting P-type ATPase in copper metabolism of hepatocyte and pathogenesis of Wilson disease (WD). METHODS: WD copper transporting properties in some organelles of the cultured hepatocytes were studied from WD patients and normal controls.These cultured hepatocytes were incubated in the media of copper 15 mg x L(-1) only, copper 15 mg x L(-1) with vincristine (agonist of P-type ATPase) 0.5mg x L(-1), or copper 15 mg x L(-1) with vanadate (antagonist of P-type ATPase) 18.39 mg x L(-1) separately. Microsome (endoplasmic reticulum and Golgi apparatus), lysosome, mitochondria, and cytosol were isolated by differential centrifugation. Copper contents in these organelles were measured with atomic absorption spectrophotometer, and the influence in copper transportion of these organelles by vanadate and vincristine were comparatively analyzed between WD patients and controls. WD copper transporting P-type ATPase was detected by SDS-PAGE in conjunction with Western blot in liver samples of WD patients and controls. RESULTS: The specific WD proteins (M(r)155,000 lanes) were expressed in human hepatocytes, including the control and WD patients. After incubation with medium containing copper for 2 h or 24 h, the microsome copper concentration in WD patients was obviously lower than that of controls, and the addition of vanadate or vincristine would change the copper transporting of microsomes obviously. When incubated with vincristine, levels of copper in microsome were significantly increased, while incubated with vanadate, the copper concentrations in microsome were obviously decreased. The results indicated that there were WD proteins, the copper transportion P-type ATPase in the microsome of hepatocytes. WD patients possessed abnormal copper transporting function of WD protein in the microsome, and the agonist might correct the defect of copper transportion by promoting the activity of copper transportion P-type ATPase. CONCLUSION: Copper transportion P-type ATPase plays an important role in hepatocytic copper metabolism. Dysfunction of hepatocytic WD protein copper transportion might be one of the most important factors for WD.

MR imaging of cerebral extraventricular neurocytoma: a report of 9 cases.

Extraventricular neurocytoma is a rare entity, most frequently occurring in brain parenchyma outside the ventricular system. The purpose of this study was to characterize the MR imaging findings in a series of 9 patients with EVN verified by results of pathologic examination. All 9 EVNs were solitary and intracranially located. Eight lesions were well demarcated, and 3 showed intratumoral hemorrhage. The solid parts of 7 tumors were primarily isointense on T1-weighted images and heterogeneously enhanced on T1WI with contrast. Although cerebral EVNs can present a wide spectrum of appearances on MR, the imaging patterns appear to vary according to anatomic location and cellularity. Lesions in frontal or parietal lobes often present as well-demarcated large masses with cystic degeneration, hemorrhage, mild-to-moderate edema, and inhomogeneous enhancement. Moreover, the general isointensity of the solid parts of EVN on T1WI may be of some specificity.

MR imaging findings of the intraspinal meningeal melanocytoma: correlation with histopathologic findings.

Our aim was to better understand and improve the accuracy of the preoperative diagnosis of intraspinal MM by a combined analysis of MR imaging and pathologic findings. All 5 patients had undergone unenhanced and contrast-enhanced MR imaging examinations. All tumor samples had immunohistochemical reactions to HMB-45, vimentin, S-100, EMA, and Leu-7 antibodies. All 5 cases were located in the intradural extramedullary compartment. Two cases had multifocal lesions, and 3 cases were solitary. Two cases showed homogeneously strong enhancement, and 3 cases showed moderate enhancement on contrast-enhanced T1WI. The tumor cells had positive reactions to HMB-45, vimentin, and S-100 antibodies. MR imaging plays an important role in the detection and diagnosis of intraspinal MM. Final diagnosis should be based on histopathology and IHC examinations.

Microvascular corrosion casting of normal tissue, transitional mucosa and adenocarcinoma in the human colon.

The microcirculatory architecture of normal tissue, transitional mucosa and adenocarcinoma of the human colon was investigated with microvascular corrosion casting (MVCC) combined with scanning electron microscopy (SEM). The study showed that the capillaries within the normal mucosa were arranged in a regular hexagonal pattern around the mucosal glands and that the microvessels of transitional mucosa mostly had lost the typical hexagonal pattern and become slightly wider in diameter. The microvessels in the tumor periphery were increased in number and disorganized, and presented large variation in morphology with claw-like formations, widened sinuses, diverticula and appendixoid patterns. Microvessels were lacking in the central areas of tumors. These morphological alterations may serve as additional indicators of tumor development.